Home Translating Report News Physicians Diseases Body Sites Lab tests Search
Home Diseases and Health Information

Background

This is a relatively common condition that usually afflicts the rim of the auricle of the ear. Trauma and sun exposure have traditionally been cited as possible inciting agents. The histopathology is characteristic, showing necrobiotic changes within the collagen and eosinophilic degenerative changes within the underlying cartilage. There is recent evidence that suggests this condition may be a maker of internal syndromes associated with microvascular injury such as diabetes mellitus and connective tissue disorders.

OUTLINE

Epidemiology  
Disease Associations  
Pathogenesis  
Laboratory/Radiologic/
Other Diagnostic Testing
 
Gross Appearance and Clinical Variants  
Histopathological Features and Variants  
Special Stains/
Immunohistochemistry/
Electron Microscopy
 
Differential Diagnosis  
Prognosis  
Treatment  
Commonly Used Terms  
Internet Links  

 

EPIDEMIOLOGY CHARACTERIZATION
SYNONYMS CNH
INCIDENCE/
PREVALENCE
Relatively common
AGE 6th decades and olfer
SEX M>F

 

DISEASE ASSOCIATIONS CHARACTERIZATION
SYNDROMES ASSOCIATED WITH MICROVASCULAR INJURY  
Chondrodermatitis nodularis helicis as a marker of internal syndromes associated with microvascular injury.

Magro CM, Frambach GE, Crowson AN.

Department of Pathology, The Ohio State University, Columbus, OH, USA.
J Cutan Pathol. 2005 May;32(5):329-33. Abstract quote  

Chondrodermatitis nodularis helicis (CNH) is held to be an idiopathic degenerative process involving the upper dermis of the auricular rim. Chondrodermatitis typically occurs in elderly men where associations with underlying trauma and sun exposure have been postulated as potential inciting triggers. Its association as a marker of systemic disease is not well established.

We describe 24 patients with CNH, in whom there were also significant underlying diseases largely associated with vascular injury including those of immune-based etiology and/or conditions which have been previously linked with granuloma annulare, another necrobiotic process of collagen. These patients with concomitant systemic disease were characteristically younger compared to the classic demographics described for CNH.

In some cases, chondrodermatitis may represent an ischemic necrobiotic disorder of collagen, potentially defining an important sign of underlying systemic disease in younger patients.
Chondrodermatitis nodularis helicis occurring with systemic sclerosis--an under-reported association?

Bottomley WW, Goodfield MD.

Department of Dermatology, Leeds General Infirmary, UK.

Clin Exp Dermatol. 1994 May;19(3):219-20. Abstract quote  

Three cases of chondrodermatitis nodularis helicis (CDNH) in patients with the limited form of systemic sclerosis are described.

Two of the three cases were noted during regular follow-up visits and the third case was found as part of a survey of the 21 other patients with systemic sclerosis routinely seen in the department.

While CDNH is known to have several predisposing causes there are no reports in the literature of this condition occurring in association with systemic sclerosis.

 

PATHOGENESIS CHARACTERIZATION

 

LABORATORY/
RADIOLOGIC/
OTHER TESTS

CHARACTERIZATION
RADIOLOGIC  
LABORATORY MARKERS  

 

GROSS APPEARANCE/
CLINICAL VARIANTS
CHARACTERIZATION
GENERAL  
Chondrodermatitis nodularis chronica helicis.

Zuber TJ, Jackson E.

Department of Family Medicine, Saginaw Cooperative Hospitals, Mich., USA.
Arch Fam Med. 1999 Sep-Oct;8(5):445-7. Abstract quote  

Chondrodermatitis nodularis chronica helicis is a painful nodule of the external ear. These uncommon lesions are most often encountered on the helix in white men older than 40 years, although they also rarely occur on the antihelix in women.

The lesions frequently present with exquisite tenderness that interferes with sleep. While the cause of this dermal inflammatory process is not known, long-term trauma or sun damage may play a role. Recurrences often complicate treatment if all sites of inflammation are not eradicated.

Surgical treatment is generally recommended, either by wide excision or by deep shave and treatment of the underlying cartilage.
Chondrodermatitis helicis: a clinical re-evaluation and pathological review.

Metzger SA, Goodman ML.
Laryngoscope. 1976 Sep;86(9):1402-12. Abstract quote  

Chondrodermatitis helicis, or painful nodule of the ear, is an uncommon benign aural lesion which is seen and treated by dermatologists and otolaryngologists. Because of the sparcity of reports in the recent literature, our experience with 50 patients over a 10-year period is presented.

The diagnosis is based on history and physical examination and biopsy need be performed only to confirm the diagnosis in atypical cases. Patients are generally middle-aged or elderly males. There are no associated systemic disorders with this condition. The lesions are discrete, grey to red in color, oval shaped with raised rolled edges, and a central ulcer or depression which often contains a crust or scale. The lesion is typically painful and tender and, for this reason, the patient seeks help shortly after the onset of symptoms. The characteristic histopathologic features are epithelial hyperplasia, collagen degeneration, focal fibrinoid necrosis, and inflammatory components.

Clinically, the lesion is misdiagnosed in the majority of instances and is presumably, therefore, treated inappropriately. It should be stressed that this is a benign condition and initial management should be aimed at conservative therapy with local steroid injection or conservative non-deforming surgery.

Wide excision, including removal of the underlying cartilage with appropriate reconstrictive closure should be reserved for the conservative treatment failures.
VARIANTS  
BILATERAL  
Bilateral chondrodermatitis helicis: case presentation and literature review.

Cannon CR.


Am J Otol. 1985 Mar;6(2):164-6 Abstract quote.  

Chondrodermatitis helicis is a benign, painful lesion of the auricle affecting the elderly population, particularly men.

These lesions usually affect the helix of the ear, although the antihelix and, less commonly, the antitragus may be involved. The lesions are most frequently unilateral, although bilateral involvement has been reported.

An unusual case of bilateral involvement of the antitragus is presented. The etiologic and pathologic manifestations are reviewed and current treatment modalities outlined.
PEDIATRIC  
Juvenile chondrodermatitis nodularis helicis: a case report and literature review.

Rogers NE, Farris PK, Wang AR.

Department of Pathology, Tulane University Health Sciences Center, New Orleans, Louisiana 70112-2699, USA.
Pediatr Dermatol. 2003 Nov-Dec;20(6):488-90. Abstract quote  

Chondrodermatitis nodularis helicis (CNH) is a disorder that affects adults. Only one case of juvenile CNH has been reported, in an 8-year-old child who suffered from dermatomyositis.

We report another child with juvenile CNH who was not afflicted with dermatomyositis or other systemic disorders. The clinical and histologic evaluations demonstrated CNH on the helix of the right ear in a 16-year-old Caucasian girl who was otherwise healthy. Serologic analysis ruled out an underlying autoimmune disorder.

We conclude that juvenile CNH is extremely rare and may occur in patients without dermatomyositis or other systemic disorders.

 

HISTOLOGICAL TYPES CHARACTERIZATION
GENERAL  
VARIANTS  
NEURAL HYPERPLASIA  
Neural hyperplasia in chondrodermatitis nodularis chronica helicis.

Cutaneous Histopathology Laboratory, Dermatology Clinic of the University Hospitals, Strasbourg, France.

J Am Acad Dermatol. 2006 Nov;55(5):844-8 Abstract quote

BACKGROUND: Chondrodermatitis nodularis chronica helicis (CNCH) is a common condition characterized by tender nodules of the helix, of uncertain mechanism. The pain is not explained by current physiopathological hypotheses.

OBJECTIVE: We sought to investigate possible nerve hyperplasia in CNCH.

METHODS: Thirty-seven cases of clinically typical CNCH were submitted to conventional microscopy after surgical excision and analyzed with immunohistochemistry using PS100 and neurofilament antibodies. As controls, we investigated 25 tumors of the ear with the same methods.

RESULTS: Large nerves (>0.1 mm in diameter) were seen in 8 of 37 cases; these were close to the cartilage or at the upper part of the ulceration. Increased numbers of small nerve sections (0.01-0.06 mm in diameter) were seen in 35 of 37 cases, with more than 20 sections per low-power field in 18 cases. Nerve hyperplasia was not observed in tumors of the ear, even in ulcerated cases.

LIMITATIONS: Hypothesis based on morphologic observations.

CONCLUSIONS: Nerve hyperplasia is present in CNCH, but is often masked by intense vascular and inflammatory reactions. This finding may explain the induction of pain by light pressure, whereas ulcerated auricular malignant tumors are generally painless. Certain authors believe that CNCH is an equivalent of prurigo nodularis, which interestingly also shows nerve hyperplasia.
PERFORATION  

Chondrodermatitis nodularis chronica helicis: a perforating necrobiotic granuloma.

Goette DK.

J Am Acad Dermatol. 1980 Feb;2(2):148-54. Abstract quote  

Seventeen cases of clinically and histologically characteristic chondrodermatitis nodularis chronica helicis (CNCH) are presented.

Thirteen histologic specimens revealed transepidermal elimination (TE) of necrobiotic material from the dermis. This elimination occurred through transepidermal channels, slits, or erosions overlying a dermal necrobiotic granuloma. The granuloma was surrounded by granulation tissue composed of histiocytes and lymphocytes. Telangiectasia and peripheral solar elastosis were common findings.

The histologic features and clinical characteristics found predominantly in white men over 40 years of age suggest that CNCH etiologically and pathogenetically represents an actinically induced perforating necrobiotic granuloma.
Chondrodermatitis nodularis helicis: a transepidermal perforating disorder.

Santa Cruz DJ.

J Cutan Pathol. 1980 Apr;7(2):70-6. Abstract quote  

Chondrodermatitis nodularis helicis is a chronic disorder occurring exclusively on the ear. It is most common in the sixth decade, and two thirds of the patients are men.

Although it has no single characteristic histologic feature, a diagnosis can be made with certainty with a clinical history and a combination of morphologic changes seen in the cartilage and overlying skin.

The natural history and histology are similar to a group of disorders characterized by transepithelial elimination known as the perforating dermatoses.

 

DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES
RELAPSING POLYCHONDRITIS  
   
PSEUDOCYST OF THE AURICLE  
Pseudocyst of the auricle: a review of 21 cases.

Lazar RH, Heffner DK, Hughes GB, Hyams VK.

Otolaryngol Head Neck Surg. 1986 Mar;94(3):360-1. Abstract quote  

Pseudocyst of the auricle (benign idiopathic cystic chondromalacia) is rare. Only 20 cases have been previously reported in the literature.

Our study retrospectively reviews 21 additional cases. We conclude that idiopathic cystic chondromalacia can occur in both sexes, in all races, and at any age. The differential diagnoses for idiopathic cystic chondromalacia are relapsing polychondritis and chondrodermatitis nodularis chronica helicis.

Recurrence of idiopathic cystic chondromalacia is uncommon following adequate local treatment.

 

TREATMENT CHARACTERIZATION
GENERAL  
Effective treatment of chondrodermatitis nodularis chronica helicis using a conservative approach.

Moncrieff M, Sassoon EM.

Department of Plastic and Reconstructive Surgery, Norfolk and Norwich University Hospital NHS Trust, Norwich NR4 7UY, UK.
Br J Dermatol. 2004 May;150(5):892-4. Abstract quote  

BACKGROUND: Chondrodermatitis nodularis chronica helicis (CNCH) is usually treated by surgical excision, but is prone to recurrence.

OBJECTIVES: To examine whether CNCH could be treated nonsurgically using a home-made, pressure-relieving prosthesis.

METHODS: A retrospective comparison was made of the outcome in 41 subjects treated surgically and 15 treated nonsurgically between 1999 and 2001.

RESULTS: Thirteen of the 15 patients (87%) treated nonsurgically were healed at follow-up after 1 month of conservative treatment and so have avoided surgery to date. In contrast, the recurrence rate of the surgically treated group was 14 of 41 (34%) patients.

CONCLUSIONS: As a result of this study, we recommend that patients presenting with CNCH be managed conservatively in the first instance.
COLLAGEN  
The treatment of chondrodermatitis nodularis chronica helicis with injectable collagen.

Greenbaum SS.

Department of Dermatology, Jefferson Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania 19107.
Int J Dermatol. 1991 Apr;30(4):291-4. Abstract quote  

Chondrodermatitis nodularis chronica helicis (CNCH) consists of a painful, erythematous, often crusted, papule most commonly found on the helical rim of the ear of white men over the age of 40 years old. It is seen less commonly in women and younger individuals, where the anatomic locations may vary to include the antihelix, antitragus, and other areas on the external ear. Over the years there have been many treatments for this disorder.

We present the use of injectable collagen implants as a conservative, effective, and practical method for treatment of this disorder.
SURGERY  
Modified surgical excision for the treatment of chondrodermatitis nodularis.

Ormond P, Collins P.

St. Vincent's University Hospital and The City of Dublin Skin and Cancer Hospital, Hume Street, Dublin, Ireland.
Dermatol Surg. 2004 Feb;30(2 Pt 1):208-10. Abstract quote  

BACKGROUND: Chondrodermatitis nodularis (CN) is a common inflammatory condition of the ear that produces a painful papule or nodule on the helix or antihelix. Excision of the cartilage alone has been demonstrated to be therapeutically and cosmetically effective.

OBJECTIVE: To simplify the surgical procedure.

METHOD: We used two steps to refine the classic technique. We excised a narrow ellipse of skin over the nodule and replaced cold steel dissection by injecting normal saline into the plane of cleavage between the skin and cartilage.

RESULTS: These two refinements resulted in maintaining the clinical and cosmetic efficacy and simplified the surgical technique.

CONCLUSION: The addition of an elliptical excision over the nodule and the use of hydrodissection in the surgical treatment of chondrodermatitis nodularis were of benefit to both the patient and the surgeon.

Treatment of chondrodermatitis nodularis with removal of the underlying cartilage alone: retrospective analysis of experience in 37 lesions.

de Ru JA, Lohuis PJ, Saleh HA, Vuyk HD.

Department of Otolaryngology/Head and Neck Surgery, UMC Utrecht, The Netherlands.
J Laryngol Otol. 2002 Sep;116(9):677-81. Abstract quote  

Most otolaryngologists treat patients with chondrodermatitis nodularis (CDN) by wedge excision. Although the results of this technique are generally good, it can leave the patient with an asymmetric, deformed ear. In the dermatological literature, a relatively straightforward technique has been described for the treatment of CDN by smoothing only the underlying cartilage. This is based on the assumption that CDN is caused by pressure necrosis of protuberant cartilage, and thus is primarily not a skin disease.

Reports on this technique claim excellent cosmetic results with only a small chance of recurrence. In the present study we analyse the application of this technique to 34 patients with 37 CDN lesions. All patients were symptom-free with a minimum follow-up of three months according to their medical reports. Seventeen patients with 19 lesions were interviewed later by telephone. In a mean follow-up of 30.7 months, 34 of these patients remained symptom-free and only one required revision surgery.

The authors recommend this safe and simple technique to other physicians who treat patients with CDN.
Chondrodermatitis nodularis chronica helicis et antihelicis.

Munnoch DA, Herbert KJ, Morris AM.

Department of Plastic Surgery, Dundee Royal Infirmary, UK.

Br J Plast Surg. 1996 Oct;49(7):473-6. Abstract quote  

A retrospective study of 50 patients (25 male, 25 female) suffering from chondrodermatitis nodularis chronica is presented. There was equal distribution between male and female, with the nodule being situated on the helix in 36 cases (23 male, 13 female), and on the antihelix in 18 cases (4 male, 14 female).

Four patients had bilateral lesions. All the patients complained of severe pain in the affected ear when they slept on it at night. Of the 54 ears in this study, 23 had undergone previous surgery for the complaint. These recurrences occurred when either skin alone, or a disproportionately large piece of skin relative to cartilage, was excised.

A treatment technique is described involving minimal skin excision combined with extensive cartilage resection. There has been no recurrence following our technique and postoperative deformity has been minimal.
The surgical management of chondrodermatitis nodularis chronica helicis.

Coldiron BM.

Department of Dermatology and Otolaryngology, University of Cincinnati Medical Center, Ohio 45267.
J Dermatol Surg Oncol. 1991 Nov;17(11):902-4. Abstract quote  

Surgical management of chondrodermatitis nodularis chronica helicis by curettage is described. The curet directs the dissection.

The necrotic cartilage is soft and is removed easily. The endpoint is reached when the curet is repelled by firm, elastic cartilage.

Contours remain normal because the skin is tented over the defect supported by the remaining cartilage.
Chondrodermatitis nodularis chronica helicis treated with curettage and electrocauterization: follow-up of a 15-year material.

Kromann N, Hoyer H, Reymann F.

Acta Derm Venereol. 1983;63(1):85-7. Abstract quote  

During the 15-year period from 1965 to 1979 a total of 142 cases of chondrodermatitis nodularis chronica helicis were diagnosed. 32% were women, a higher proportion than in earlier materials.

The treatment was principally curettage followed by electrocauterization. 78 patients were re-examined after an average lag of 7.1 years. The relapse rate was 31%.

This simple surgical technique seems equal to the more elaborate procedures in respect of recurrence rate and more satisfactory from a cosmetic point of view.

Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.


Commonly Used Terms

Basic Principles of Disease
Learn the basic disease classifications of cancers, infections, and inflammation

Commonly Used Terms
This is a glossary of terms often found in a pathology report.

Diagnostic Process
Learn how a pathologist makes a diagnosis using a microscope

Surgical Pathology Report
Examine an actual biopsy report to understand what each section means

Special Stains
Understand the tools the pathologist utilizes to aid in the diagnosis

How Accurate is My Report?
Pathologists actively oversee every area of the laboratory to ensure your report is accurate

Got Path?
Recent teaching cases and lectures presented in conferences


Internet Links

Pathologists Who Make A Difference
Search for a Physician Specialist


Last Updated November 14, 2006

Send mail to The Doctor's Doctor with questions or comments about this web site.
Read the Medical Disclaimer.

Copyright The Doctor's Doctor